Download Single Coronary Artery from Single Sinus in Complete Transposition

Cas e R e po r t
Single Coronary Artery from Single Sinus in
Complete Transposition of Great Artery: An
Exceptional Case
Sudeep Pathak1,
Rajeev Gupta2,
Altaf Masood3
MD. PGDC: Intensive Care Specialist and Non Invasive Cardiologist with Max Neonatal Pediatric
Intensive Care Hospital and Narmada Trauma & Emergency Center, Bhopal, 2MD. DM. Associate
Professor, Gandhi Medical college, Bhopal, 3MD. Pediatrician & Intensive Care Specialist at with Max
Neonatal Pediatric Intensive Care Hospital, Bhopal
1
Corresponding Author: Dr. Sudeep Pathak, MD PGDC, 3 Nupur Kunj, E-3 Arera Colony,
Bhopal - 462016, Madhya Pradesh. Mobile: 09893837104. Phone: 0755-2465466,
E-mail: [email protected]
Abstract
Complete Transposition of great arteries (D TGA) characterizes by Ventriculo-arterial discordance, along with AV concordance.
In D-TGA, Aorta arises from morphologic right ventricle and Pulmonary artery arises from Left ventricle. In new born and
infants this is a potentially lethal form of heart disease. The incidence of D-TGA is 1 in 2300 to 1 in approximately 5100 live
birth. Ninety percent of d TGA are associated with dual sinus origin of coronaries, in this singular case,the baby had single
sinus origin of coronary artery. In this exceptional study, a new born baby girl of 16 day age who could not maintain her oxygen
saturation, found to have complete transposition of aorta, which was associated with single sinus origin of her coronary artery
which indicates that both coronary arteries arise from one of the two but not both of the facing sinuses by a single ostium. Her
left and right coronary and left circumflex artery arises from single coronary artery. She was taken for anatomic correction by
arterial switch operation with re-implantation of coronary artery and she made excellent recovery. This case highlights that we
have to be very careful to assess the coronaries thoroughly in all cases of d TGA as many cases of ischemia, infarct and LV
failure have been documented in patients who were not treated appropriately with respect to coronary anatomy.
Keywords: Complete transposition of great arteries, Corrected transposition of great artery, D-TGA, Simple transposition
INTRODUCTION
Complete transposition or D-TGA is one the most fatal
form of congenital cyanotic heart disease if remain
untreated. Malformation consist of the origin of the
Aorta from morphologic right ventricle and that of the
pulmonary artery from the morphologic left ventricle.1
Consequently, pulmonary and systemic circulation is
connected in parallel rather than in series connection. In
one circuit, the Systemic venous blood passes through
the right atrium, right ventricle, and then to aorta. In the
other, pulmonary venous blood passes through left atrium,
left ventricle and to the pulmonary artery. This situation
is incompatible with life unless mixing of the two circuits
occurs.
The incidence of D-TGA is 1 in 2300 to 1 in approximately
5100 live birth.2 The malformation represents 5-8 % of
congenital cardiac malformations but accounts for 25 %
International Journal of Scientific Study | June 2014 | Vol 2 | Issue 3
of death from congenital heart disease in the first year
of life.3 Male outnumber females by a ratio of 4:1,4 and
seldom occurs in first born infants, but a twofold increase
in incidence occur in mother who have had three or more
pregnancy.
Associated congenital anomalies which are seen with D
TGA are Patent foramen ovale, septum secundam atrial
septal defect, ductus arteriouses, pulmonary stenosis, aortic
arch anomalies such as hypoplasia, coarctation, interruption
of aorta and aortic arch, or tricuspid and mitral valve
abnormalities.
CASE REPORT
A baby girl of 16 day old,born through nor mal
uncomplicated delivery, got admitted on 29 September
2013 with history of respiratory distress, chest infection
94
Pathak, et al.: Single Coronary Artery from Single Sinus in Complete Transposition
and bluish discoloration of lips, fingers and tongue
since birth. Baby is third sibling of her parent. Baby was
consulted by her parents with pediatrician during the past
15 day and was given antibiotics for the same.
Subsequently baby got admitted as there was no
improvement in her respiratory distress and cyanosis.
On examination her heart rate was 142 per minute, oxygen
saturation was 40%, blood pressure was 90/60 mmHg
respiratory rate was 44/minute, her birth weight was
3.6 kg there was presence of central cyanosis, on CVS
examination she had central cyanosis, with full volume
bounding pulse. Precordial palpation was normal, loud
palpable second heart sound at the left base is felt. Mid
systolic murmur heard over aortic area, mid diastolic flow
murmurs of mitral and tricuspid origin5 were auscultated.
Loud and single second heart sound of anterior place aorta
was auscultated too.
Echocardiography reveals aorta arising from right ventricle,
pulmonary artery arising from left ventricle with “sausage”
appearance in short axis (Figures 1 and 2). The great arteries
appear as double circles with aorta anterior and to the right
of the main pulmonary artery. The anterior aortic root and
posterior pulmonary trunk run parallel to each other and
do not cross. There was small Patent ductus arteriouses
which was about to close with left to right shunt and peak
gradient of 13 mmHg.
There was single coronary artery arising from single sinus
of left aortic sinus and gave rise to right coronary artery,
left coronary artery and circumflex artery. Usually in more
than 90% of DTGA, there is dual sinus origin of coronary
artery (Figure 3).6,7
There was no evidence of associated other congenital
anomaly.
Chest X ray showed narrow aortic knuckle, increased
pulmonary vascularity, and fairly normal size cardiac
silhouette.
MANAGEMENT
She was treated with oxygen supplementation 3 liter/
hr, fluids and antibiotics, along with this baby was given
Prostaglandin E1 to keep the duct patent so that baby
could overcome the crises till she get her surgery done,
subsequently she was taken for surgery. Although, Baloon
atrial septostomy (BAS) was considered for the patient but in
view of potential complications of BAS such as neurologic/
embolic stroke8,9 and poor exercise performance10,11 after
95
BAS in comparison to Arterial switch, the baby was taken
for Arterial switch operation. Although there are reports of
intra operative and post operative coronary artery kinking,
ischaemia and lV dysfunction12,13 but overall it gives the
best possible result so the baby had undergone Arterial
switch and made good recovery after the surgery and got
discharged postoperatively (Figures 4-7).
DISCUSSION
Complete transposition of great arteries are one of the
rare congenital heart disease of new born and in DTGA,
chances of three coronary artery arising from single aortic
sinus is less than 10%. To suspect complete transposition
of great arteries in a new born, pediatric cardiologist or
pediatrician should have vast knowledge to detect even
minute details of the baby profile, gender, and clinical
presentation. It should be aggressively confirmed with
cardiac Doppler and Chest X-ray. The clinical recognition
of D-TGA is based on following features (1) Male third
or fourth child, (2) large birth weight (3) Cynosis in the
neonatal period (4) Radiological feature of increased
blood flow in presence of cyanosis and egg-shaped cardiac
silhouette with a narrow vascular pedicle and absent thymic
shadow (5) Echocardiographic identification of Aortic
alignment with morphologic Right ventricle and Pulmonary
alignment with morphologic left ventricle (Ventricular
arterial discordance) and right atrial alignment with right
ventricle and left atrium alignment with left ventricle
(Atrioventricular concordance).
Some communication between the two circulations must
exist after birth to sustain life; otherwise unoxygenated
systemic venous blood is directed inappropriately to
systemic circulation and oxygenated pulmonary venous
blood is directed to the pulmonary circulation. Almost
all patients have an interatrial communication such as
Patentductusarteriouses, Ventricular septal defect or Atrial
septa defect.
There could be various mode of coronary artery origin
in DTGA, common amongst them are Dual sinus origin,
in which left coronary artery arise from left aortic sinus
which in turn give rise to left anterior descending artery
and circumflex artery and right coronary from posterior
aortic sinus. Dual sinus origin accounts for 90% of cases.
Single sinus origin indicates that both coronary arteries arise
from one of the two but not both of the facing sinuses by
a single ostium or multiple ostia. In this exceptional study,
there was single coronary artery from left aortic sinus
which in turn gave rise to Left coronary, right coronary
and circumflex arteries. The incidence of this among the
DTGA is less than 10%.
International Journal of Scientific Study | June 2014 | Vol 2 | Issue 3
Pathak, et al.: Single Coronary Artery from Single Sinus in Complete Transposition
Figure 1: Echocardiography of transposition seen in long axis
with great arteries side by side
Figure 4: Transposition of great Artery (TGA)- great artery cut
open
Figure 5: Single coronary artery arising from left aortic cusp
with its branches- LCA, RCA, Lcx
Figure 2: Echocardiography of great arteries in short axis view
in DTGA with sausage appearance
Figure 6: TGA with single coronary artery giving rise to LCA,
RCA, Lcx
Figure 3: D-TGA in apical four chamber view with pulmonary
artery arising from left ventricle and bifurcating into right and
left pulmonary artery. Operative pictures of Arterial switch
with single coronary artery and its branches {Left coronary
artery-left main (LCA) , Right coronary artery (RCA) and left
circumflex artery (Lcx)}
Management of D TGA could be done by Mustard and
senning atrial switch procedures, but in view of increasing
frequency of complications such as dysarrythmias, sudden
cardiac death and right ventricular dysfunction has lead to
go for anatomic correction with Arterial switch operation.
Long term patency and growth of the coronary arteries are
International Journal of Scientific Study | June 2014 | Vol 2 | Issue 3
Figure 7: Post operative repair of TGA with coronary and its
branches
96
Pathak, et al.: Single Coronary Artery from Single Sinus in Complete Transposition
crucial for the arterial switch operation to be considered
the procedure of choice for the surgical management of
TGA. Although intraoperative and post operative coronary
artery kinking, occlusion can occur resulting in ischaemia
and ventricular dysfunction.
2.
CONCLUSION
6.
Complete transposition of great arteries is one of the
uncommon Cyanotic congenital heart diseases and single
coronary sinus origin for all three coronary in DTGA
is even exceptionally singular. Diagnosis of Complete
transposition of great arteries should be suspected with the
gender, para of pregnancy with its clinical presentation. It
should be aggressively confirmed with echocardiography.
Management has to be done aggressively with the idea
to keep the shunt patent (Patent ductus arteriouses,Atrial
septal defect or ventricular septal defect) with the help
of Prostaglandin E1. Subsequently, whenever possible
anatomical correction should be done with Arterial switch.
7.
3.
4.
5.
8.
9.
10.
11.
12.
REFERENCES
1.
De La CruzMV, Arteaga M, EspinovelaJ, et al. Complete Transposition
of the great arteries: Types and morphogenesis of ventriculoarterial
discordance. Am heart J. 1981;101,271.
13.
Gutgesel HP, Garson A, Namara DG. Prognosis for the newborn with
transposition of the great arteries. Am J Cardiol. 1979;44,96.
Liebman J, Cullum L, Belloe NB. Natural history of the transposition of the
great arteries. Circulation. 1969; 40:237.
Flyer DC, Report of the New England regional infant cardiac program.
Pediatric. 1980; 65(supl)377.
Well B: The sounds of murmur in transposition of the great vessels. Br
Heart J. 1963; 25:748.
Yoo S, Burrows P, Moes F et al. Evaluation of coronary artery patterns
in complete transposition by laid back aortography. Cardiol Yoaung.
1996;6:149.
Sim EK, Van son JA, Edward WD, et al. Coronary artery anatomy
in complete transposition of the great artery. Ann Thoracic surgery.
1994;(4):890-4.
Applegate SE, Lim DS. Incidence of stroke in patients with d – transposition
of great arteries that undergoes balloon atrial septostomy in the University
Health system Consortium Clinical data base/resource manager. Catheter
Cardiovasc Interv. 2010;(76):129-131.
Mukherjee D,Lindsay M,Zhang Y, et al. Analysis of 8681 neonates with
transposition of great arteries: Outcome with and without Rashkind balloon
atrial septostomy. Cardial young 2010;(20):373-380.
Sterret LE, Ebenorth ES Montgomery GS, et al. Pulmonary limitation to
exercise after repair of d transposition of the great vessel: Atrial baffle
versus arterial switch. Pediatr cardiol. 2011;(32):910-916.
Daebritz SH, TieteAR, Sachweh JS, et al. Systemic right ventricular failure
after atrial switch operation: Midterm results of conversion into an arterial
switch. Ann Thorac Surg. 2001;(71):1255-1259.
Oztunc F, Bari, Scedil S, Adaletli I et al. Coronary events and anatomy after
arterial switch operation for transposition of great arteries: Detection by
16 row multislice computed tomography angiography in pediatric patients.
Cardiovasc Intervent Radiol 2009;(32):206-212.
Angeli E, Formigari R, Pace Napoleone C et al. Long term coronary artery
outcome after arterial switch operation for transposition of the great arteries.
Eur Cardiothoracic Surg. 2010;(38):714-720.
How to cite this article: Sudeep Pathak, Rajeev Gupta, Altaf Masood. "Single Coronary Artery from Single Sinus in Complete
Transposition of Great Artery: An Exceptional Case". Int J Sci Stud. 2014;2(3):94-97.
Source of Support: Nil, Conflict of Interest: None declared.
97
International Journal of Scientific Study | June 2014 | Vol 2 | Issue 3